Urinary alpha-ketoglutarate is elevated in patients with hyperinsulinism-hyperammonemia syndrome.
D000293:Adolescent; D000328:Adult; D002675:Child, Preschool; D005260:Female; D008401:Gas Chromatography-Mass Spectrometry; D005969:Glutamate Dehydrogenase / Q000235:genetics / Q000378:metabolism; D006801:Humans; D022124:Hyperammonemia / Q000235:genetics / Q000652:urine*; D006946:Hyperinsulinism / Q000235:genetics / Q000652:urine*; D007223:Infant; D007656:Ketoglutaric Acids / Q000652:urine*; D008297:Male; D008875:Middle Aged; D009154:Mutation / Q000235:genetics / Q000502:physiology; D012016:Reference Values; D013577:Syndrome
D007656:Ketoglutaric Acids; D005969:Glutamate Dehydrogenase